POS1570 LONG-TERM SAFETY AND EFFECTIVENESS OF CANAKINUMAB IN FAMILIAL MEDITERRANEAN FEVER (FMF) – 36-MONTH DATA FROM THE RELIANCE REGISTRY
Authors: J. Henes, N. Blank, T. Krickau, A. Pankow, I. Foeldvari, F. Dressler, G. Horneff, T. Kallinich, P. Oommen, F. Meier, F. Weller-Heinemann, B. Kortus-Goetze, M. Hufnagel, J. Rech, I. Andreica, J. Weber-Arden, J. B. Kuemmerle-Deschner
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Familial Mediterranean Fever (FMF) is characterized by recurrent attacks of fever, serositis, and elevated laboratory parameters (CRP, SAA). According to EULAR recommendations, treatment goals are the control of acute attacks and subclinical inflammation as well as the improvement of patient´s quality of life. In a phase 3 pivotal study (CLUSTER study), FMF patients have been successfully treated with the interleukin-1β inhibitor canakinumab (CAN). <h3>Objectives</h3> The RELIANCE registry explores the long-term efficacy and safety of CAN under routine clinical practice conditions in pediatric (age ≥2 years) and adult patients with FMF and other recurrent hereditary fever conditions. <h3>Methods</h3> RELIANCE is a prospective, non-interventional, observational study that enrolls patients with clinically confirmed diagnoses of FMF routinely receiving CAN. Clinical data, physician assessments and patient-reported outcomes are assessed at baseline and 6-monthly intervals. <h3>Results</h3> This interim analysis includes baseline as well as 18- and 36-month data from N=96 FMF patients (CAN-pretreated: N=71, CAN-naïve: N=20) enrolled by December 2022. Median age in this cohort was 21.5 years (2−61 years) and the proportion of female patients was 46%. At baseline, median duration of prior CAN treatment was 1.0 years (0−6 years). The MEFV genotype has been documented in 67 patients. Among 53 (79%) carriers of pathologic MEFV variants, 17 were homozygous, 6 heterozygous plus a second pathologic variant, 8 heterozygous plus a non-pathologic variant, and 22 heterozygous plus wild type. At month 36, disease activity was mild (3 and 2 out of 10) according to patients rating and lab parameters. Physician assessment indicated disease remission in 60% of the patients (53% at baseline) and 57%/14% of the patients having no/mild-moderate disease activity. The majority of patients reported no impairment of social life (100%, 75%) nor negative impact on mood (88%, 100%) due to the disease. Disease activity parameters decreased in the observation period, particularly in patients without prior CAN therapy (Table 1). In total, 22 serious adverse events (SAE) were reported, of which 3 were classified as drug related. <h3>Conclusion</h3> Interim data from the RELIANCE study, the longest running real-life CAN registry confirm efficacy and safety of long-term CAN treatment in FMF patients. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> Jörg Henes Consultant of: Novartis, AbbVie, Sobi, Roche, Janssen, Boehringer-Ingelheim, Grant/research support from: Novartis, Roche, Norbert Blank Consultant of: Novartis, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Actelion, UCB, Boehringer-Ingelheim, Roche, Grant/research support from: Novartis, Sobi, Tobias Krickau Speakers bureau: Novartis, Consultant of: Novartis, Grant/research support from: Novartis, Anne Pankow: None declared, Ivan Foeldvari Consultant of: Novartis, Frank Dressler Consultant of: Abbvie, Mylan, Novartis, Pfizer, Grant/research support from: Novartis, Gerd Horneff Speakers bureau: AbbVie, Bayer, Chugai, Merck Sharp &amp; Dohme, Novartis, Pfizer, Roche, Grant/research support from: AbbVie, Bayer, Chugai, Merck Sharp &amp; Dohme, Novartis, Pfizer, Roche, Tilmann Kallinich Speakers bureau: Roche, Prasad Oommen Grant/research support from: Novartis, Florian Meier Speakers bureau: Novartis, Frank Weller-Heinemann: None declared, Birgit Kortus-Goetze Consultant of: Novartis, Markus Hufnagel Grant/research support from: Novartis, Jürgen Rech Speakers bureau: AbbVie, Biogen, BMS, Chugai, GSK, Janssen, Lilly, MSD; Mylan, Novartis, Roche, Sanofi, Sobi, UCB, Consultant of: AbbVie, Biogen, BMS, Chugai, GSK, Janssen, Lilly, MSD, Mylan, Novartis, Roche, Sanofi, Sobi, UCB, Grant/research support from: Novartis, Sobi, Ioana Andreica Speakers bureau: Abbvie, Chugai, Novartis, UCB, MSD, Lilly, Sobi, Astrazeneca, Amgen, Pfizer, Gilead, Paid instructor for: Astrazeneca, UCB; Consultant Abbvie, Chugai, Novartis, UCB, Galapagos, Takeda, Astrazeneca, Lilly, Boehringer Ingelheim, Amgen, Sobi, Julia Weber-Arden Employee of: Novartis, J. B. Kuemmerle-Deschner Consultant of: Novartis, AbbVie, Sobi, Grant/research support from: Novartis, AbbVie, Sobi.